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PPOM 1 Lecture 136: Intro to Immunologically Mediated Disease
Primary Biliary Cholangitis (Primary Biliary Cirrhosis) Diagnosis and Treatment
Diagnosis
Positive Antimitochondrial Antibodies
Increased Alkaline Phosphatase (ALK-P)
Increased Cholesterol
Treatment
Ursodiol
Liver Transplant
2 mins
Ulcerative Colitis Associations
Associations
Toxic Megacolon
Colorectal Carcinoma
Anterior Uveitis
Pyoderma Gangrenosum
Ankylosing Spondylitis
Primary Sclerosing Cholangitis
P-ANCA
1 min
Ulcerative Colitis Characteristics
Autoimmune
Mucosal and Submucosal Only
Rectal Involvement
Stepwise Lesions
Friable Pseudopolyp
Crypt Abscess
Loss of Haustra
Lead Pipe Appearance
Bloody Diarrhea
2 mins
Post Streptococcal Glomerulonephritis
Pathophysiology
Nephritic
Children
Signs and Symptoms
Edema
Cola Colored Urine
Follows Group A Streptococcal pharyngitis or Skin Infection
Diagnosis
Neutrophils
Immunofluorescence (IF) Granular
Lumpy Bumpy Appearance on Immunofluorescence (IF)
Subepithelial Immune Complex Humps
2 mins
Rapidly Progressive Glomerulonephritis
Pathogenesis
Nephritic Syndrome
Crescent Shape
Crescents Consist of Fibrin and C3b
With Parietal Cells, Monocytes, and Macrophages
IMMUNOFLUORESCENCE
Linear Immunofluorescence
Negative Immunofluorescence
Granular Immunofluorescence
Etiology
Goodpasture Syndrome
Granulomatosis with Polyangiitis (Formerly Wegener's)
Microscopic Polyangiitis
Prognosis
Poor Prognosis
3 mins
Pemphigus Vulgaris
Pathophysiology
Autoantibodies against desmosomes
Symptoms
Flaccid Bullae on Skin
Oral Mucosa Involvement
Intraepidermal Acantholysis
Fluid Loss
Infection
Nikolsky Positive
Diagnosis
Biopsy showing Autoantibodies
Treatment
IVIG and Plasmapheresis
Oral Steroids
Immunosuppressants
2 mins
Primary Immune Thrombocytopenia
Characteristics
Following Infection or Vaccination
Antiplatelet Antibodies
Clinical Features
Often Asymptomatic
Bleeding
Petechiae
Diagnosis
Thrombocytopenia
Increased Megakaryocytes
Management
Corticosteroids
Intravenous Immunoglobulin (IVIG)
Rituximab
Splenectomy
4 mins
Thrombotic Thrombocytopenic Purpura (TTP)
Inhibition or deficiency of ADAMTS 13
vWF Multimers are Not Degraded
Microthrombi (and Emboli)
Symptoms
FAT RN Pentad
Fever
Anemia
Thrombocytopenia
Renal Dysfunction
Neurologic Abnormalities (Altered Mental status)
Treatment
Plasmapheresis
Corticosteroids
Splenectomy
2 mins
Antiphospholipid Syndrome
Characteristics
Antiphospholipid Antibodies
Hypercoagulability
Clinical Features
Recurrent Thromboembolisms
Recurrent Miscarriages
Diagnosis
Anticardiolipin Antibodies
Anti-Beta-2-Glycoprotein Antibodies
Lupus Anticoagulant
Management
Anticoagulation
2 mins
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